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Autoimmune Pulmonary Alveolar ProteinosisNCT06546098

Molgramostim Nebulizer Solution Expanded Access Program Protocol

A study testing Molgramostim nebulizer solution in people with Autoimmune Pulmonary Alveolar Proteinosis, run by Savara Inc. at 12 sites in United States.

Available

Status not confirmed. The sponsor has not verified this record for over two years, so it may be out of date.

Official record
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Trial summary

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disorder in which a material called surfactant builds up in the lungs and makes it hard to breathe. In addition to shortness of breath, people with aPAP can experience persistent cough, overwhelming fatigue, unintentional changes in weight, chest or back pain… Read more about this study

Who is it for?
Ages 18 years and older
What is being tested?
Molgramostim nebulizer solution
How many people
Not given
Where
12 sitesUnited States
Study dates
Start not givento not given
Phase
Expanded access
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 18 years and older
  • All sexes
  • Adult, Older adult

Who may be able to join

  • Eligible patients must:
  • Be ≥18 years of age at the time of signing the informed consent.
  • Agree to use a highly effective form of contraception (see Section 3.5).
  • Have a positive serum anti-granulocyte macrophage colony-stimulating factor (GM CSF) autoantibody test result confirming aPAP.
  • Have a history of pulmonary alveolar proteinosis (PAP), based on examination of a lung biopsy, bronchoalveolar lavage (BAL) cytology, or a high-resolution computed tomogram (HRCT) of the chest.
  • Have at least one symptom of aPAP, including but not limited to dyspnea (at rest or with exertion), cough, or fatigue.
  • Be capable of giving signed informed consent which includes compliance with the requirements and restrictions listed in the informed consent form (ICF) and in this protocol.
  • Be willing and able to comply with the visit schedule and treatment plan specified in the protocol, as judged by the physician.

Who cannot join

  • Eligible patients must not:
  • Have a diagnosis of hereditary or secondary PAP, or a metabolic disorder of surfactant production.
  • Require a whole lung lavage (WLL) at the time of screening (patient may be eligible 1 week post WLL).
  • Have received GM-CSF treatment within 1 month prior to the screening visit.
  • Have been treated with any investigational product within 5 half-lives or 3 months (whichever is longer) prior to the screening visit.
  • Have a history of allergic reactions to GM-CSF or any of the excipients in the nebulizer solution.
Show all 11Show fewer
  • Be using significant (e.g., more than 10 mg/day systemic prednisolone) immunosuppression.
  • Have a history of severe and unexplained side effects during aerosol delivery of any medicinal product.
  • Have a history or current diagnosis of a myeloproliferative disease or leukemia.
  • Have any other medical condition which in the opinion of the physician would make the patient unsuitable for treatment with molgramostim nebulizer solution.
  • Be pregnant or breastfeeding, or planning to become pregnant during treatment with molgramostim nebulizer solution.

Trial locations and contacts

12 sites in 1 country.

Yasmine Wasfi, MD., Ph.D. contact

512-851-1364

yasmine.wasfi@savarapharma.com

Michele Rhee, MBA, MPH contact

617-807-0488

michele.rhee@savarapharma.com

12 sites in 1 country

United States12 sites
  • University of Maryland School of Medicine

    Baltimore, Maryland, 21201

    Valeriya Rodionova, MD, 410-328-2835, vrodionova@som.umaryland.edu

    Available
  • The Medical University of South Carolina

    Charleston, South Carolina, 29425

    Zerlinna Teague, 843-792-0965, recruitment@musc.edu

    Available
  • University of Virginia Health

    Charlottesville, Virginia, 22902

    Heather M Haughey, Ph.D., 434-243-4008, UVAJEDITrials@uvahealth.org

    Available
  • Cincinnati Children's Hospital Medical Center

    Cincinnati, Ohio, 45229

    Brenna Carey, MS, PhD, 513-636-8916, Brenna.Carey@cchmc.org

    Available
  • UT Southwestern Medical Center

    Dallas, Texas, 88915

    Uma Gude, uma.gude@utsouthwestern.edu

    Available
  • Duke University Hospital

    Durham, North Carolina, 27708

    Wendy Curry, 919-668-8281, Wendy.curry@duke.edu

    Available
  • University Florida Health

    Gainesville, Florida, 32610

    Ali Ataya, MD, Ali.Ataya@medicine.ufl.edu

    Available
  • University of California

    Los Angeles, California, 90095

    Maria C Granone, MD, CCRP, mgranone@mednet.ucla.edu

    Available
  • AGH Allegheny General Hospital

    Pittsburgh, Pennsylvania, 15212

    Marvin R Balaan, MD, FCCP, 412-463-3298, marvin.balaan@ahn.org

    Available
  • WakeMed Raleigh

    Raleigh, North Carolina, 27610

    Kevin Davidson, MD

    Rhonda Norton, CCRC, 919-350-5252, rnorton@wakemed.org

    Available
  • UW Medicine

    Seattle, Washington, 98195

    Lisa Batten, 206-310-7517, lsetya@uw.edu

    Available
  • Washington University School of Medicine

    St Louis, Missouri, 63110

    Kristine Kempf, RN, 314-273-8131, kempf@wustl.edu

    Available

Other trials in United States

About this study

Official title: Expanded Access to Inhaled Molgramostim Nebulizer Solution in Adults With Autoimmune Pulmonary Alveolar Proteinosis (aPAP)

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disorder in which a material called surfactant builds up in the lungs and makes it hard to breathe. In addition to shortness of breath, people with aPAP can experience persistent cough, overwhelming fatigue, unintentional changes in weight, chest or back pain, suddenly feeling out of shape, and general discomfort.

Currently, there are no approved medications for aPAP in the United States, but the symptoms of aPAP can be treated with whole lung lavage (WLL). WLL is an invasive procedure that temporarily removes surfactant, and it can result in serious consequences like trauma to the lung, a collapsed lung, and prolonged requirement for artificial ventilation.

Read more

Savara is studying an investigational drug called molgramostim nebulizer solution to see if it activates the cells that help clear surfactant from the lungs, which improves oxygen transfer from the lungs to the bloodstream. Molgramostim nebulizer solution is administered by inhalation using a hand-held nebulizer. In clinical trials, molgramostim nebulizer solution has shown improvements in gas exchange and patient reported outcomes.

This expanded access program will make molgramostim nebulizer solution available to adult patients with diagnosed aPAP. Access must be obtained through the treating physician. Patients will dose molgramostim nebulizer solution 300 micrograms (mcg) once daily and be followed by their physician every 3 months to assess their clinical status and report any adverse events.

Read the detailed description

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease mediated by autoantibodies targeting granulocyte macrophage colony-stimulating factor (GM-CSF), resulting in malfunctioning macrophages with impaired surfactant catabolism. The latter causes accumulation of surfactant in the alveoli, which has a negative impact on gas exchange between lung and blood. The clinical course of autoimmune PAP varies among patients with continuously slowly progressive disease in most patients, spontaneous improvement in a small percentage (5-7%), and rapid progression and/or pulmonary fibrosis, respiratory failure, and death in others. The most common cause of death is respiratory failure followed by secondary pulmonary bacterial infections. Estimated 5-year mortality rates vary between 10-30%, with overall disease-specific survival rates at 5 years exceeding 80%.

There are currently no approved pharmacological treatments for aPAP in most of the world. Whole lung lavage (WLL) is the primary treatment option currently available for most aPAP patients. However, its invasive nature, limited access, and the variable effect of WLLs due to lack of standardization emphasize that there is an unmet need for a non-invasive, safe and well-tolerated, easily accessible and effective treatment for aPAP patients.

The rationale for treating aPAP patients with molgramostim nebulizer solution is based on the capacity of GM-CSF to promote differentiation and mobilization of different myeloid leukocyte subsets including neutrophils, tissue macrophages/dendritic cells or their circulating precursors. It is crucially involved in anti-microbial pulmonary host defense and ameliorates lung injury by increasing the size and activation of the alveolar macrophage pool. GM-CSF also contributes to the proliferation of megakaryocytic and erythroid progenitors and plays a key role in surfactant homeostasis, by maturation of alveolar macrophages.

Molgramostim is a non-glycosylated recombinant human granulocyte macrophage colony stimulating factor (rhGM-CSF) produced by using recombinant DNA technology via a bacterial (E. coli) expression system. Molgramostim is formulated in a sterile nebulizer solution (molgramostim nebulizer solution) which is supplied in vials containing 300 µg of molgramostim in 1.2 mL solution and is administered by inhalation via an investigational eFlow Nebulizer System (PARI Pharma GmbH).

Results of a completed randomized, placebo-controlled clinical trial support the safety, tolerability, and efficacy of inhaled molgramostim nebulizer solution as a treatment for autoimmune PAP patients.

Being tested
Molgramostim nebulizer solution
Keywords
surfactant accumulation, autoimmune, alveolar proteinosis, GM-CSF
Medical subject headings
Pulmonary Alveolar Proteinosis, Acquired, Pulmonary Alveolar Proteinosis

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • Molgramostim nebulizer solutionDrug

    Also called Recombinant human granulocyte-macrophage colony stimulating factor (rhGM-CSF)

    Solution for inhalation

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted9 Aug 2024

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Expanded access
IDs, dates and publications
ClinicalTrials.gov ID
NCT06546098
Sponsor's protocol ID
SAV006-06
Status last verified
Jul 2026
First submitted
5 Aug 2024
Last updated
26 Oct 2026
Results posted
No

Publications

  • Bogunovic M, Ginhoux F, Helft J, Shang L, Hashimoto D, Greter M, Liu K, Jakubzick C, Ingersoll MA, Leboeuf M, Stanley ER, Nussenzweig M, Lira SA, Randolph GJ, Merad M. Origin of the lamina propria dendritic cell network. Immunity. 2009 Sep 18;31(3):513-25. doi: 10.1016/j.immuni.2009.08.010. Epub 2009 Sep 10.

    PubMed 19733489
  • Hamilton JA. Colony-stimulating factors in inflammation and autoimmunity. Nat Rev Immunol. 2008 Jul;8(7):533-44. doi: 10.1038/nri2356.

    PubMed 18551128
  • Inaba K, Inaba M, Romani N, Aya H, Deguchi M, Ikehara S, Muramatsu S, Steinman RM. Generation of large numbers of dendritic cells from mouse bone marrow cultures supplemented with granulocyte/macrophage colony-stimulating factor. J Exp Med. 1992 Dec 1;176(6):1693-702. doi: 10.1084/jem.176.6.1693.

    PubMed 1460426
  • Khan A, Agarwal R. Pulmonary alveolar proteinosis. Respir Care. 2011 Jul;56(7):1016-28. doi: 10.4187/respcare.01125. Epub 2011 Apr 15.

    PubMed 21496372
  • Kumar A, Abdelmalak B, Inoue Y, Culver DA. Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach. Lancet Respir Med. 2018 Jul;6(7):554-565. doi: 10.1016/S2213-2600(18)30043-2. Epub 2018 Feb 1.

    PubMed 29397349
  • McCarthy C, Avetisyan R, Carey BC, Chalk C, Trapnell BC. Prevalence and healthcare burden of pulmonary alveolar proteinosis. Orphanet J Rare Dis. 2018 Jul 31;13(1):129. doi: 10.1186/s13023-018-0846-y.

    PubMed 30064481
  • McCarthy C, Carey BC, Trapnell BC. Autoimmune Pulmonary Alveolar Proteinosis. Am J Respir Crit Care Med. 2022 May 1;205(9):1016-1035. doi: 10.1164/rccm.202112-2742SO.

    PubMed 35227171
  • Metcalf D. The molecular biology and functions of the granulocyte-macrophage colony-stimulating factors. Blood. 1986 Feb;67(2):257-67.

    PubMed 3002522
  • Park LS, Friend D, Gillis S, Urdal DL. Characterization of the cell surface receptor for human granulocyte/macrophage colony-stimulating factor. J Exp Med. 1986 Jul 1;164(1):251-62. doi: 10.1084/jem.164.1.251.

    PubMed 3014035
  • Trapnell BC, Carey BC, Uchida K, Suzuki T. Pulmonary alveolar proteinosis, a primary immunodeficiency of impaired GM-CSF stimulation of macrophages. Curr Opin Immunol. 2009 Oct;21(5):514-21. doi: 10.1016/j.coi.2009.09.004. Epub 2009 Sep 30.

    PubMed 19796925
  • Trapnell BC, Nakata K, Bonella F, Campo I, Griese M, Hamilton J, Wang T, Morgan C, Cottin V, McCarthy C. Pulmonary alveolar proteinosis. Nat Rev Dis Primers. 2019 Mar 7;5(1):16. doi: 10.1038/s41572-019-0066-3.

    PubMed 30846703
  • Trapnell BC, Inoue Y, Bonella F, Morgan C, Jouneau S, Bendstrup E, Campo I, Papiris SA, Yamaguchi E, Cetinkaya E, Ilkovich MM, Kramer MR, Veltkamp M, Kreuter M, Baba T, Ganslandt C, Tarnow I, Waterer G, Jouhikainen T; IMPALA Trial Investigators. Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis. N Engl J Med. 2020 Oct 22;383(17):1635-1644. doi: 10.1056/NEJMoa1913590. Epub 2020 Sep 7.

    PubMed 32897035

Questions about this trial

What is this clinical trial studying?

A study testing Molgramostim nebulizer solution in people with Autoimmune Pulmonary Alveolar Proteinosis, run by Savara Inc. at 12 sites in United States.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 8 inclusion and 11 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: status not confirmed, as of the sponsor's last update on 26 Oct 2026. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 12 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

Are results available for this study?

Results are not available in the source record.

How can a doctor refer a patient or contact the research team?

Contact Yasmine Wasfi, MD., Ph.D. (512-851-1364, yasmine.wasfi@savarapharma.com), or a site near the patient. AzTrials does not enrol participants or take referrals.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT06546098, which also lists 12 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 26 Oct 2026.

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